Noncirrhotic portal vein thrombosis (PVT) is a rare disease that usually presents with small nonfatal "herald bleeding" with low mortality. Classic findings of noncirrhotic PVT include esophageal varices, splenomegaly, a normal liver, and an organized thrombus of the portal vein. We present a case of previously unreported sudden unexpected death from noncirrhotic PVT in an asymptomatic elderly woman, review the literature of this entity, and examine the pathophysiology of the formation of fatally ruptured varices. The portal vein must be carefully examined in all cases where there is no coexisting cirrhosis.